Renal cystic disease: the role of the primary cilium/centrosome complex in pathogenesis

Abstract

Cystic diseases of the kidney are among the most frequent inherited disorders and constitute a significant cause of end-stage renal disease. Identification of the genes involved in these disorders and their protein products has provided key insights into the cellular processes that underlie cyst development and mediate disease progression. Of particular note, the proteins implicated in these disorders localize to the cilia/centrosome complex and have focused recent research on the role of this complex in the pathogenesis of renal cystic disease. Unraveling the spatial and functional relationship between these cystoproteins and the cilia/centrosome complex will undoubtedly provide a better understanding of the pathogenesis of cystic diseases and potentially offer windows for therapeutic intervention.